Thesis Research Paper of Dr. Prahlada N.B
Study of Clinico-pathological and Radiological Spectrum of Cholesteatoma in Children and Correlation of Computerized Findings with Surgical Findings.
II Review of Literature
III Aims and Objectives
IV Materials and Methods
VII Summary and Conclusions
- Master Charts
BE Bony erosion
CP Central perforation
FCD Facial canal dehiscence
LSCS Lateral semicircular canal
MCD Mastoid cortex dehiscence
OD Ossicular destruction
PAA Post-aural abscess
PCW Posterior canal wall
SD Soft tissue density mass
SPD Sinus plate destruction
Study of Clinico-pathological and Radiological Spectrum of Cholesteatoma in Children and Correlation of Computerized Findings with Surgical Findings.
SUBMITTED IN PARTIAL FULFILMENT OF THE REQUIREMENTS
FOR THE DEGREE OF
M. S. (E. N. T.)
POSTGRADUATE INSTITUTE OF MEDICAL
EDUCATION AND RESEARCH, CHANDIGARH
JULY, 1995 PRAHALADA N.B.
This is to certify that the work contained in this thesis entitled, "STUDY OF CLINICO PATHOLOGICAL AND RADIOLOGICAL SPECTRUM OF CHOLESTEATOMA IN CHILDREN AND CORRELATION OF COMPUTERIZED TOMOGRAPHIC FINDINGS WITH SURGICAL FINDINGS" has been carried out by Dr. Prahlada N.B. under our direct supervision and guidance.
All investigations in connection with this work have been carried out by the candidate himself and are genuine.
Dr. Suresh C. Sharma
Department of ENT,
Postgraduate Institute of Medical Education and Research,
Dr. Naresh K. Panda
Dr. Rajiv Bapuraj
l wish to express my profound gratitude to my teacher Dr. Suresh C Sharma, Additional professor, Department of ENT, PGIMER, Chandigarh for the timely help and guidance given to me.
I wish to express my deep gratitude to Prof. S. B. S. Mann, Chief, Department of ENT, PGIMER, Chandigarh for his parental attitude and the unstinting support.
I am indeed grateful to Dr. Naresh K Panda, Assistant Professor, Department of ENT, for his support and valuable suggestions, not only in this study but otherwise as well.
I am thankful to Dr. Rajeev Bapuraj, Assistant professor, Dept of Radiology, whose every helping nature and advice was a source of inspiration at all stages of my work.
I thank all the little patients who have participated in this study, but for their cooperation this study would have been impossible.
I thank Mr. Sharma and Sunil Sharma for their expert assistance in the preparation of this work.
I thank all my Senior residents and fellow collegues for their cooperation and constructive criticism.
l would like to thank my parents for what / am today.
PRAHLADA N. B.
Dedicated to my teachers, parents and little patients
Otological experience has shown that the incidence of aural cholesteatoma in children is much lower compared to cholesteatoma in adults, the ratio of cholesteatoma in adults to children being 5.6:1(1) and 4:1 (2) . Aural cholesteatoma in children poses many challenges to the otologist by being a rapidly growing disease which is more extensive within a well pneumatized mastoid bowl (2, 3, 4).
Making the diagnosis of cholesteatoma in children is often a difficult task due to paucity of symptoms, the difficulty in examination, and their small, tortuous ear canals. The triad of otorrhoea, hearing loss and abnormal otoscopic findings should raise the possibility of cholesteatoma. The presence of congenital cholesteatoma presents another challenge in the diagnoses of pediatric cholesteatoma, which often presents as a white mass behind an intact eardrum.
The complications of chronic suppurative otitis media are commoner in the young individuals. In fact, younger the patient, the more likelihood of developing intracranial complications.
The subject of greatest debate among the otologist relates to the choice of surgical approach, which must provide; a disease free ear and serviceable hearing required for the development of child. the successful treatment of cholesteatoma in the paediatric age group has been achieved by using the same basic principles of therapy used for cholesteatoma in adults. The canal-wall-down approach is associated with a large mastoid cavity and a need for regular follow up and cavity care. The canal-wall-up approach which avoids a large mastoid cavity is associated with disadvantages of greater incidence of residual disease in the mesotympanum and recurrence of cholesteatoma in children. However, this problem can be overcome by a planned two stage canal-wall-up procedure, i.e. modified canal-wall-up procedures.
Inspite of such new surgical techniques, the recidivism of cholesteatoma is higher in children than compared to adults.
Radiologists have found that, the interpretation of the extent of cholesteatoma in children is difficult. 'Buckingham and Valvassori' have experienced discrepancies in 6 of the total of 1 1 cases in their pioneering study of tomography of temporal bone.
In the developed countries, the advent of powerful antibiotics, timely and precise surgical intervention by otologists has reduced the morbidity and mortality of cholesteatoma considerably. The same is not true for the developing countries like India, where vast areas of land are devoid of primary health services, let alone specialized otological facilities. Thus cholesteatoma is a major health problem with significant morbidity, particularly in paediatric age group.
Since a considerable number of children with acquired cholesteatoma attend the otolaryngology services of our hospital, it was felt necessary to study in detail the clinical spectrum of cholesteatoma in paediatric age group with regards to clinical presentation, radiological assessment and surgical findings and their respective follow-up in the post-operative period.
REVIEW OF LITERATURE
Paediatric cholesteatoma is of significant importance to the otolaryngologist as it poses many challenges. Firstly, the otologist must make a correct and an early diagnosis. Secondly, the otologist must provide a disease free ear that will remain safe throughout adulthood and can be easily followed up. Thirdly, a serviceable hearing level, which is important for the development of normal language and communication skills, should be achieved. Fourthly, the otologist should educate the patient and the family as to the nature of the disease, the need for long term follow-up, and the possibility of further radiographic studies, reconstructive surgery and aural rehabilitation.
As such the cholesteatoma itself is still a most intriguing pathological entity since the days of Johannes Mueller, who coined the term 'Cholesteatoma' in 1828, its remarkability in children further presents the otolaryngologist with a variety of diagnostic and therapeutic challenges.
Earlier studies on paediatric cholesteatoma have reported inconsistent results, which makes it difficult to fully understend the nature of this disease. Pioneering studies of Tos, Jahnke, Pavla et al and Glassock et alt, (4,7,8), propose that cholesteatoma is rapidly growing and more aggressive in children than in adults. Reudi (9) suggests that there is a connective tissue layer in the mastoid of young children that helps to promote cholesteatoma matrix growth. Similarly, the growth of the mastoid and the surrounding cranium may have some effect on the growth factor in cholesteatoma (10). Recently it has been proposed that cholesteatoma is less extensive disease with fewer complications, contrary to popular belief (11).
Majority of children have acquired cholesteatoma. Acquired cholesteatoma can be classified into two categories. The most common form is primary acquired cholesteatoma, which arises from a skin lined retraction pocket, within which retained keratin debris accumulates. This is also known as Attic retraction cholesteatoma (12), and is usually confined to the region of pars flaccida.
The pathogenesis of attic retraction cholesteatoma is the subject of much research and debate. There are four basic theories of development.
In invagination theory, Whittmack proposed that attic block caused by persistence of hyperplastic embryonic type of mucoperiosteum in the epitympanum result's in negative pressure in the attic. The negative pressure then causes retraction of Shrapnell's membrane into the attic, where keratin debris collects and cholesteatoma develops. Ruedi, Nager and Lange (9,13,14) in their basal cell hyperplasia theory state that, cone like extensions from the basal layer of epidermis can become invasive as a result of infection, Prickle cells grow into the subepithelial layer through breaks in the basal lamina, creating cones of epithelium or microcholesteatomas. Habermann (15, 5) proposed that following perforation of Shrapnell's membrane, epithelium grows into epitympanum, much like a secondary acquired cholesteatoma. This invasion ultimately reaches barriers that ca use the invading edges to meet and create a cyst or sac like structure. Keratin accumulation then causes progression of the disease. This theory is known as epithelial invasion theory and is supported by temporal bone studies of Palva et a1 (7) . Studies by Sade (16) tend to indicate that middle ear mucosa has the potential to transfornn into keratinizing squarnous epithelium Mucosal metaplasia may be triggered by infection, inflammation, and certain chemicals.
Irrespective of theories of pathogenesis, most common presenting symptoms of primary acquired cholesteatoma are: Hearing loss, Otorrhoea, Otalgia, tinnitus and vertigo. Most common finding at examination of ears are, a retraction pocket either in the ear-drum or extending toward the mastoid in 70% of children, followed by a tympanic membrane performation in one third and very few (8%) with a white mass behind the tympanic membrane i.e. congenital cholesteatoma.
In secondary acquired cholesteatoma, in growth of stratified squamous epithelium from the meatus to line the denuded tympanic cavity, epitympanum and antrum, following acute necrotic otitis media with sloughing of the tympanic mucoperiosteum results in cholesteatoma. This cholesteatoma offers a favourable culture medium for various pathogens and putrefactive bacteria from external auditory meatus, producing the characteristic foul smelling discharge. The microorganisms commonly found on culture are mixed, which include pseudomonas aeruginosa, proteus, enterobacter, Staphylococci, aerobic and anaerobic nonhaemolytic streptococci, diphtheroid bacilli and aspergillus molds.
The presence of congenital growth poses another challenge in the diagnosis of paediatric cholesteatoma, which presents with a mass behind the intact eardrum. Congenital cholesteatoma -tend to originate in the anterior epitympanum and over time, grow into the petrous apex without producing the symptoms until the patient in adult life develops hemifacial spasms (5, 17).
Previous theory of pathogenesis of congenital cholesteatoma postulated that failure or resorption of small masses of epidermal rests in the dorsolateral epitympanum of a human fetus could proliferative to form a cholesteatoma of the middle ear (18). This theory was not pursued and later the "Epidermoid formation theory" based on study of fetal temporal bones state that, squamous cell rests are identifiable from 10 to 33 weeks of gestation in the anterior superior lateral wall of the tympanic cavity, which resulted in cholesteatoma This epidermoid formation is present at histological differentiation point demarcating the transition from simple cubiodal epithelium lining the tympanic cavity and eustachian the anterior to it (19) . After 33 weeks of gestation the formation is not usually present.
Though radiological studies are a valuable aid in the diagnosis of ear diseases associated with changes in osseous structure, there is a conflict of opinions concerning the role of radiology in the management of cholesteatoma, a relatively common and potentially dangerous disease of the middle ear cleft. On the one hand, there is the opinion of Smyth (20): "In many otologic centres X-ray pictures of the petrous bones are made traditionally in every case of chronic otitis media. This practice constitutes an indispensable waste of money and time and does not in any way assist a competent surgeon. Has any surgeon who needs X-ray to show him whether there is cholesteatoma, and if so to indicate its dimensions, the right to sit behind an operating microscope. On the other hand, Dulac et al (21) contradict this statement by saying, "At present it is not possible to explore a chronic otitis with or without a cholesteatoma without employing tomography. These two extreme view points serve to illustrate the wide disagreement on this topic.
The history of radiology (22) of cholesteatoma dates back to 1905, when Schuller described the first view to visualize pathologic lesions in the area frequently involved in chronic ear disease, namely, "attic-aditus C antrum" or the "key area". This involves lateral skull view with an elevation of the beam to 30 degrees and gives a good exposure of the antrum and part of the attic. A portion of the head of the malleus may be seen, but the remainder of the ossicles are obscured by the dense bony labyrinth. The landmarks of temporal bone, the root of the zygoma, the condyle of the mandible, the temporomandibular joint, the external auditory canal superimposed on the tympanic cavity, the vestibule of the labyrinth and internal auditory canal, the cellular structure of the, mastoid process, the sinus plate and the tegmen plate are well visualized.
In 1920, Law (23) described the view which bears his name, in which the X-ray beam is directed caudally from a 15 degree elevation and anteriorly by 15 degrees. This is still a single most important radiologic projection in the management of cholesteatoma. The extent of pneumatization and the position of the sinus plate and dural plates are well visualized. However, ossicles are not visible since they are superimposed on the dense arcuate eminence of the superior vertical semicircular canal.
Many other variations which came into vogue later, are only used rarely in special circumstances to visualize the areas which are not visible in Law's view. Primary Mayer's view (24) is similar to Schuller's view, but X ray beam is still further elevated to depress the labyrinth and explore the cellular area in the antrum, aditus and attic. The head of the malleus and part of the incus are clearly seen in the Primary Mayer's view. In the conventional (usually referred to simply as the Mayer's projection), the same 40 degree elevation of the beam is maintained, but in addition, the head is rotated so that the face turns away from the centre by about 30 degrees. This rotation of the beam effectively frees the "key areas" from the dense shadow of the labyrinth. The malleus and incus are well seen in the upper tympanic and epitympanic cavities and the cellular pattern of the osseous outer attic wall is well indicated. But, the antrum is less well visualized because it is partially obscured by the arcuate eminence, and the obliquity of the Mayer's projection produces a distortion that may confuse the surgeon.
To lessen this distortion, the Owen's position (25) has been devised. The elevation of the beam is reduced to 30 degrees, but the rotation of about 30 degrees is maintained. All the anatomic landmarks of the Mayer's position, are retained but the malleus and incus are not visualized. The lateral wall of the attic with its pneumatic pattern is clearly visible and the antrum is in a more "normal" position, just above the arcuate eminence.
In patients who have been previously operated on the type of the operation can be identified in the Mayer and Owen positions. After simple mastoidectomy, the posterior canal wall and ossicles are intact. after modified radical operations, the ossicles are present but the posterior canal wall is absent. After radical surgery, both the ossicles and the posterior canal wall may be seen to have been removed.
Stenver's view (26) is taken with central ray depressed, with a rotation of the face toward the centre so as to bring the petrosa perpendicular to beam. This projection is best for the antrum, cellular structure of the petrous apex, internal auditory canal and vestibule of the labyrinth. The position also shows the tegmen plate, tympanic cavity, ossicles, condyle of the mandible and mastoid air cells lateral to the antrum and in the mastoid tip.
The third projection of Chausse (Chausse III) (22) is a modified frontal projection useful for the study of the attic, aditus and mastoid antrum and especially of the lateral wall of the attic. This wall run from back to front and slightly outward, forming an angle of 10 and 15 degrees with sagittal plane of the skull.
The Chamberlain-Towne's view (22) and Submentovertex views are used to view the petrous apex and mastoid antrum but have no advantage over the Stenver's position, with the disadvantage of loss of clarity and detail because of increased antrum-to-film distance. Therefore, these positions are not used routinely.
In the typical case of chronic otitis with cholesteatoma the infection develops in a poorly pneumatized temporal bone and the disease process is limited to the tympanic cavity, epitympanic cavity, aditus and antrum. The bone of the mastoid process is usually sclerotic and the few cell wall very thick. The first evidence of bone destruction is the loss of the normal osseous pattern of the attic. As the disease progresses, the aditus is widened and finally antral enlargement can be demonstrated. In the opinion of most radiologists an enlargement of the mastoid antrum is the most important and indispensable feature in the radiological diagnosis of cholesteatoma of the middle ear. According to McMillan (27), the normal sized antrum is 6 mm wide by 10 mm high in a sclerosed mastoid. Any increase in size of the antrum has been considered to be due to erosion of the bone by growing cholesteatoma. However, very small, as well as large antra may occur as normal variants. These findings may also be caused by granulation tissue as well as cholesteatosis. A condensation of the periantral bone is considered as additional evidence. Cholesteatoma itself casts no shadow and its presence can only be inferred from the bone erosion it produces. Consequently cholesteatoma which do not produce bone erosion cannot be diagnosed by conventional radiology. Winderen and Zimmer (28) classified cholesteatoma radiologically depending on their size into three groups: Large ("bean-sized" and larger), small ("pea-sized" and smaller) and medium (the size between these two extremes).
Large cholesteatoma may cause erosion of the dural plate or sinus plates with or without sequestration, which can be visualized in Law's or Shullers views. Erosion of semicircular canal sufficient to produce a labyrinthine fistula may sometimes be demonstrated in the Stenver's view and Chausse III projection (22).
It is difficult to diagnose recurrent cholesteatoma on plain X-rays in patients who have keen previously operated on unless serial films are available showing progressive enlargement of the cavity.
Congenital cholesteatoma of the middle ear produces a sharply demarcated area of bone destruction with a sclerotic margin but without evidence of chronic ear disease. Valvossori (6) considers that there are two criteria which distinguish a cholesteatoma of the middle ear cleft which has congenital rather than acquired origin. these are:
a) An intact ear drum with no evidence of a perforation.
b) An intact spur with erosion of the attic walls higher up and not involving the site of attachment of the eardrum, giving a scooped out appearance of outer attic wall.
Inspite of these observations, the diagnosis of cholesteatoma by conventional radiography has evaded eyes of many observers. In Waltner's (29) series attic cholesteatoma remained undetected in X-ray film studies and the diagnosis of antral cholesteatoma was extremely inaccurate. McMillan (27) found bone destruction of the temporal bones in 45% of 288 cases of surgically confirmed cholesteatoma with Law's projection and in 60% of cases when using Towne's projection, whereas Holmes (1938) and Chat and Kittredge (1958) noted destruction in 50% of cases were same projections were employed. Bert Jensen et al used Schuller, Runstrom III and Chausse III projections in 85 cases and found destruction in about 62% of cases. By combining a number of standard projections Winderen and Zimmer (28) made a definitive diagnosis of cholesteatoma in only 25% of their cases and found indications of cholesteatoma in another 50%(14).
The chances of detecting cholesteatoma by conventional radiography is still smaller in children. The mastoid in children with acquired cholesteatoma were reported as pneumatized in 54%, one half of these having clouding of the air cells. In 46% the mastoid was observed to be sclerotic. Majority of patients with congenital cholesteatoma have normal mastoid pneumatization, only a few cases have poor pneumatizartion and clouding due to associated effusion as an exception (30).
Conventional X-ray tomography was a further development in radiological diagnosis of diseases of the inner and middle ear. The IL tomography is a method of examining body organs and structures by serial sections obtained in various planes. With conventional X-ray tomography, sections of various thickness are obtained by blurring out objects above and below the desired plane. In such a system, during the exposure, film and Xray focus move in opposite directions with a constant ratio between their velocities and the film describing a translatory motion in relation to the object (22, 30).
The linear tomography employs simplest type of movement, which also has the shortest exposure time. The linear is most suitable for the larynx, but less suitable for petromastoid (30).
Complex motion of the X-ray tube, either hypocycloidal or spiral, gives the most uniform blurring of structures outside the plane of the section, as well as producing thinner sections of 1-2 mm thickness. The polytome continues to be used for routine demonstration of the petrous temporal none, as this gives the best and most convenient demonstration of bone detail, especially of internal auditory meatus, more cheaply and with less radiation to the patients eyes than either plain films or computerized tomography (30) .
Usually anteroposterior tomographic views are taken for the diagnosis of cholesteatoma and it affords sufficient information about the lesions in the attic and antrum. It is only in the event of special complications, in particular facial palsy, that lateral views are obtained in order to visualize better the canal of the facial nerve (31).
The anteroposterior projection shows the characteristic structures in the anterior part of the tympanic cavity. In normal radiography, the superomedial lip osseous part of the external auditory canal is termed the "Radiological spur". Projecting upwards from this spur there is the lateral wall of attic, another very important landmark. In addition, Medially, the promontary with cochlea can be seen and just above this the second part of the canal for the facial nerve (31). The tegmen tympani is often entirely absent or only faintly visible on normal tomograms. Thus its absence is not necessarily a sign of pathological destruction. Antrum and the semicircular canals can be demonstrated in slightly posterior cuts.
The tomographic diagnosis and evaluation of the extent of cholesteatoma is based on the detection of bony erosion and soft tissue changes in the middle ear and mastoid. Of the two findings only the first is reliable, since the radiographic density of a cholesteatoma is same as that of granulation tissue and other soft tissue masses 3l . With the help of tomography surgeon can detect before surgery extensive erosion of the lateral attic wall, absence of the lateral attic wall, erosion of the posterior external auditory canal, the status of the ossicles, labyrinthine involvement, facial canal erosion, extension into the petrous apex, exposure of the sigmoid sinus and tegmen, and unexpected anatomic variations and anomalies of the temporal bone, such as an anomalous deviation in the course of the facial nerve, a high jugular bulb into the hypotympanum and an aberrant internal carotid artery (32).
The tomographic criteria for the diagnosis of cholesteatoma is based on a study by Buckingham a& Valvossori (6) , who correlated otoscopic findings with tomographic findings and made following observations :
The characteristic radiographic pattern for pars flaccida cholesteatoma consisted of the following :
a. There was erosion of the anterior portion of the lateral wall of the attic in 27 of the 30 cases.
b. Erosion of anterior tympanic spine in 22 of the 30 cases.
c. Though medial displacement of the head of malleus with or without erosion seen only in 10 of the 30 cases, this finding was pathognomonic for cholesteatoma of the pars flaccida.
Tomographic findings in pars tensa cholesteatoma particularly involving posterosuperior marginal perforation were
a. The posterior portion of the lateral wall of the attic was eroded in 15 of 27 cases.
b. Erosion of long process of the incus in 22 of the 27 cases.
c. In 8 of 27 cases, the head of the malleus and body of the incus were displace laterally, and this was pathognomonic of pars tensa cholesteatoma.
The radiographic pattern of the findings in combined perforations was combination of the patterns described for the perforations of the pars tensa and pars flaccida.
Later they correlated these findings with surgical findings of cholesteatoma and found that the tomographs accurately predicted the extent of the surgical lesion in most of the cases. 80 of the 100 cases of cholesteatoma correlated well. In the remaining 11 cases, the extent of the cholesteatoma into the antrum and mastoid could not be accurately predicted. Five of these failures were in large pneumatic mastoids in which the cholesteatoma had infiltrated into the pneumatic cells but had not yet begun to destroy the cell system. The tomographs, however, did show clouding of the mastoid cells without cell wall destruction.
Brunner and Sandberg (31), in their series of 90 cases of cholesteatoma found that, distended antral cavity in 71 % of cases, smooth walled cavity in 58% of cases, destruction of the lateral attic wall in 51 % of the cases, absence of tip of the spur in 46% of the cases, destruction of the tip of the spur in 16% of the cases, destructive changes in the medial wall of the tympanic cavity in 21 % and absence of entire ossicles in 37%. A Berret et a1 (33), in their series of 25 children, found destruction of the radiological spur in only 60% of the cases and were able to diagnose cholesteatoma in only 80% of the cases using both conventional radiography and tomography.
Though standard linear or pluridirectional tomography was aimed at improving visualisation of localized areas of the body. A radiological image derived from these methods had at least two limitations from point of view of diagnostic specificity (30). They are:
a) Presence of background blur or structures above and below the focal plane.
b) A relatively large proportion of the detected radiation scattered from the patient.
Even if these two limitations could be reduced to "acceptable" levels, conventional films and screens are inherently nonuniform and relatively less sensitive than computerized tomography, and would limit subject contrast 3% to 5% which is greater than the range for some tissue.
Therefore, the advent of computerized tomography heralded a new dimension in the diagnosis of the middle and inner diseases which mitigate the observational limitation of conventional x-ray tomography.
High resolution CT was a further step in this direction which opened a new vista to various explorers to study and demonstrate the detail anatomy of temporal bone. various structures that were previously poorly visible on conventional radiography and pluridirectional tomography are made clearly visible by HRCT. However, complex structural relationships within the temporal bone cannot be visualized on single plane, making understanding of the CT anatomy of the temporal bone challenging and difficult. Though many planes are possible and have been tried, only two planes, axial and coronal planes are used for the temporal bone. The axial plane, also called transverse or horizontal plane can be obtained in CT with the patient in a comfortable position and the gantry is tilted slightly to adjust the scan parallel to the nasion-binaural plane or orbitomeatal plane. The examination area extends to between the first turn of the cochlea and the superior semicircular canal. Axial plane is an ideal tomographic plane for the baseline study of the temporal bone and provides patient comfort, the possibility of comparing both ears and the only disadvantage is the radiation dose received by lens of the eyes. The coronal plane is obtained with patient in the prone position and the head tilted upwards. The gantry is tilted until the scan plane is perpendicular to thee nasion binaural or orbitomeatal plane or parallel to ramus of the mandible. The examination area extends to between the anterior margin of the attic and the posterior semicircular canal (32, 34).
Many authors have studied HRCT anatomy of the temporal bone of the live patients, cadavers and dry skulls with intact ossicles and have correlated this with microdissections of temporal bones (35, 36).
The computerized tomographic evaluation of the acquired cholesteatoma is based on the detection of a non-dependent, homogenous soft tissue mass with a focal area of bone destruction. Of the two findings the second one is reliable since radiographic density of a cholesteatoma is the same as that of granulation tissue and other soft tissue masses (6). However, in HRCT, the granulation tissue has higher computed tomography attenuation values than cholesteatoma and often can be differentiated from the cholesteatoma. Complete opacification of the middle ear with no bony destruction makes radiologic differentiation of cholesteatoma from middle ear effusions and granulations tissue is often difficult. The presence of the an air fluid level or a soft tissue (fluid) mass in the dependent portion of the middle ear would render support for a diagnosis of effusion. On occasion, aggressive infections such as active chronic suppurative otitis media or soft tissue tumours may mimic cholesteatomas by causing erosion of bone or ossicular displacement.
In the attic following signs indicate the cholesteatoma (38):
a) Destruction of the scutum i.e., lateral spur of the bone formed by the junction of the lateral boundary of the attic and the roof of the external auditory canal. And this is the earliest sign.
b) Bone destruction in the lateral attic wall.
c) Destruction of the ossicles.
d) Erosion of the medial attic wall.
This is a less common sign but may lead to involvement of the facial canal or a labyrinthine fistula.
Complete or partial destruction of the malleus and incus and sometimes displacement medially or laterally by the cholesteatoma may be demonstrated, but the most unusual findings is a slight downward displacement of the malleus. Demonstration of this displacement and of the cholesteatoma as a clearly defined mass are possible with HRCT.
Cholesteatoma in the antrum is characterized by cavity formation. These cavities are smooth walled. A cavity with irregular boundaries is usually due to chronic otitis with osteitis. It is important to differentiate a cavity due to a cholesteatoma from normal mastoid antrum.
Mahammood P. Mafee et a1 (39) correlated CT findings with that of pluridirectional tomography and concluded by saying, "though tomography is superior in demonstrating subtle bony changes, the superior contrast resolution of CT makes it the method of choice in the demonstration of the soft tissue masses". Chat Virapongse 40 et al did similar correlation, but they continued to advocate tomography and recommended both CT and pluridirectional tomography in conjunction to study middle ear soft tissue masses. Pluridirectional tomography for its accurate diagnosis of osseous abnormalities and CT for soft tissue masses.
Inspite of the advent of high resolution computerized tomography, the tomographic interpretation of the extent of cholesteatoma in children is difficult. Buckingham and Valvassori 6 have found six of the total of 11 cases of discrepancies in tomographic prediction of the extent of the lesion in children.
Reports of one series (41) state that cholesteatoma involved the ossicles less frequently in children, whereas others (7) reported that the ossicles were most vulnerable in the paediatric acquired cholesteatoma erosion of the incur occurred most frequent (78%) followed by involvement of the malleus (54%) and the stapes (40%) (10). These authors also state that, in comparison to adult cholesteatomas, complications occurred less often in the paediatric group. Whereas, others have experienced more complications in younger individuals. And few have followed an adage, "Younger the patient, the more likely is he/she to develop an intracranial complication" (42).
The subject of greatest debate among otologists relates to choice of surgical approach. Sheehy, Jansen and Glasscock (1, 3, 43) have advocated Canal-wall-up mastoidectomy, whereas Canal-wall-down mastoidectomy has gained acceptance by Jahnke and Palve (4, 7). Others espouse a flexible attitude, selecting the surgical procedure after taking into account several factors: The location and extent of the disease, the clinical assessment of eustachian tube function, the mastoid size and pneumatization and the degree of involvement of the ossicles.
The functional status of the eustachian tube was estimated indirectly by the appearance of the middle ear space, the condition of the pars tensa, the pneumatization of the mastoid bone, impedance audiometry and presence of either nasal obstruction or adenoid hypertrophy.
Canal-wall-down mastoidectomy tended to be performed in the children with the most extensive disease, poor eustachian tube function and sclerotic mastoids. Canal-wall-up procedures were used in children who generally had localised disease and clinically had adequate eustachian tube function and few with minimal disease in mesotympanum required only tympanotomy. In contrast, the children with congenital cholesteatoma who were generally younger tended to have disease localized to the middle ear and frequently could be treated with an extended tympanotomy.
Primary reconstruction of the ossicular mechanism has been endorsed by most of the authors as a basic principle. Occasionally, reconstruction was done in states with extensive disease, poor eustachian tube function, and no middle ear space.
A higher rate of recidivism was experienced by authors who favoured a single surgical approach. Sheehy and Glasscock (1, 33) reported 25% and 5% of recidivism with single approach i.e., canal-wall-up approach. The results are same with those who used only canal-wall-down technique. Low failure rate was experienced by those who adopted a philosophy of treating each cholesteatoma individually and selecting the most appropriate procedure that will assure the eradication of the disease completely, as dictated by the operative findings. Most authors unanimously opine that children with acquired cholestesatoma treated on the average have worse hearing than in the overall pediatric series, probably because of the influence of better hearing levels observed in the congenital subgroup. In general, the children who had good hearing preoperatively tended to mintain their hearing after surgery, while those who had poor hearing preoperatively had only slight improvements.
The above studies demonstrate that the nature of pediatric cholesteatoma is still incompletely understood. And further studies needs to be done to understand the nature, early means of detecting children who are at risk of forming cholesteatoma so that the methods can be devised to try to prevent this disease. Though the surgical treatment of cholesteatoma in the pediatric age group can be achieved by using the same basic principles of therapy used for cholesteatoma in adults, it is anticipated that improved surgical techniques will evolve so that recidivism will be decreased, hearing results improved and postoperative care minimized.
AIMS AND OBJECTIVES
1. To study the clinico-pathological and radiological spectrum of cholesteatoma in children.
2. To correlate the computerized tomography findings with operative findings.
MATERIALS AND METHODS
This study consisted of 25 children below 14 years of chronic, suppurative, otitis media of unsafe type requiring mastoid exploration, admitted with the Otolaryngology services of the Nehru Hospital , PGIMER, Chandigarh .
A clinical proforma filled up for each patient incorporating details regarding particulars of the patient, history, clinical examination and investigations, including bacteriological examination of ear discharge. All patients ears were examined under microscope during outpatient otology special clinic and before surgery under operating microscope. Hearing status was assessed by play audiometry or pure tone audiometric examination according to the age and compliance of the patient.
Radiological investigation consisted of both conventional plain radiography and computerized tomography. Conventional plain radiography in the form of a lateral oblique view (Law's) of both ears. In computerized tomography, high resolution serial 2 mm thick sections were obtained in both axial and coronal planes. Axial images were obtained parallel to the orbitomeatal plane. Coronal sections were done in scanning angle that is parallel to verticle ramus of the mandible.
All patients underwent mastoid exploration and the type of surgery was determined by the otological diagnosis and intra-operative findings. The type and extent of disease was studied during surgery.
All patients were followed up in ENT out patient department after 6 weeks and 12 weeks of surgical exploration to determine the state of the mastoid cavity.
A total of 25 patients, below the age of 14 years with chronic suppurative otitis media and cholesteatoma requiring mastoid exploration were treated in ENT Department of PGIMER, Chandigarh within the period of 18 Months (from January 1994 to June 1995). The detailed information regarding age, sex, clinical findings, radiological findings, intraoperative findings and outcome of the patients is given in master charts.
Table I: Sex Distribution (n=25)
No. of Patients
Of the 25 patients, 14 (56%) were boys and 11 (44%) were girls.
Chart. 1: Distribution by Sex (n=25).
Table II: Age Distribution (n=25)
|Age Group||No. of Cases||Total||Percentage (%)|
|Group I: 0 - 2 years||0||0||0||0%|
|Group II: 2.1 - 5 years||2||1||3||12%|
|Group III: 5.1-8 years||1||0||1||4%|
|Group IV: 8.1 - 11 years||6||2||8||32%|
|Group V: 11.1 - 14 years||5||8||13||52%|
All patients were divided into 5 age groups. Group 1: 0-2 years of age, group II : 2.1-5 years of age, group III : 5.1-8 years of age, a group IV 8.1-11 years of age and group V : 11.1-14 years of age. There were no patients .in group I : 0-2 years of age and the youngest patient was 5 years of age. Male predominance was seen in patients aged upto 11 years (Fig. 2). Girls marginally out-numbered boys in the age group 11. 1-14 years (Fig. 2).
Fig. 3: Distribution by Age.
Table III: Distribution by involved ears (n= 25)
No. of Cases
Of the 25 cases, 7 (28%) patients had disease only in right ear, 13 (52%) patients had disease only in left ear and the remaining 5 (20%) patients had disease in both ears.
5 patients with bilateral ear disease, the ear with active, serious and extensive disease was operated on first (Fig. 3).
Fig. 3: Distribution by involved ears.
Table IV: Symptoms Distribution (n=25)
No. of Patients
Commonest presenting complaint was otorrhoea (100%) followed by hearing loss (88%) and tinnitus (12%). 4 (16%) patients presented with post-aural abscess and only one patient with vertigo. Patients with post aural abscess also complained of earache.
Table V: Distribution by duration of complaints (n=25)
Duration in years
No. of Patients
|0 - 3 years||8||32%|
|3.1 - 6 years||7||28%|
|6.1 - 9 years||3||12%|
|9.1 - 12 years||4||16%|
|> 12 years||3||12%|
15 patients had duration of complaints of less than 6 years. 8 of these patients were symptomatic for 3 years or less. four patients had complaints since their first year of life. Average duration of complaints was 6.9 years (Fig. 4).
Fig. 4: Distribution by duration of Complaints.
Table VI: Distribution by Ear findings (n=25)
No. of Patients
|SITE OF CHOLESTEATOMA|
|Posterior canal wall sagging||2||8%|
|Mass behind an intact drum||0||0%|
On microscopic examination of the diseased ear, presence of retraction pocket was the commonest finding. 48% had retraction in the posterosuperior region, 24% of the cases had attic retraction and remaining 12% of the cases had anterosuperior retraction pocket. 8 (32%) patients had associated tympanic membrane perforation. 2 of these 8 patients had subtotal perforation and remaining small central perforations. The commonest site of cholesteatoma was posterosuperior region.2 patients, had posterior canal wall sagging and therefore tympanic membrane could not be visualized. Granulations, polyp and recurrent cholesteatoma in operated cavity were encountered in one patient each. There were no patients with cholesteatoma behind an intact drum.
Table VIIa: Distribution by Pre-operative hearing loss
Type of Hearing loss
No. of Patients
Table VIIb: Level of Hearing loss
No. of Cases
|0 - 10 dB||5||20%|
|11 - 20 dB||2||8%|
|21 - 30 dB||6||24%|
|31 - 40 dB||11||44%|
All patients had pure conductive loss, except one patient who had a mixed loss with 30 dB air-bone gap (Table 7a). Only one fifth of the patients (20%) had minimal hearing loss and almost half of the patients (44%) had moderate conductive loss with 31-40 dB air-bone gap. Only one patient had severe conductive loss with 44 dB AB gap (Fig. )
Table VIII: X- ray Law's lateral oblique view
No. of Cases
Majority (60%) X-rays of involved ear showed sclerotic mastoid. 36% of the cases showed a lytic lesion and one X-ray showed an operated cavity mimicking a lytic lesion.
Computerized Tomographic findings
Table IX: Evidence of Cholesteatoma
Evidence of Cholesteatoma
No. of Cases
|Soft tissue density mass alone||3||12%|
|Soft tissue density mass + Bony erosion||20||80%|
Preoperative computed tomography diagnosis of cholesteatoma was made in 23(92%) cases. The hall mark of cholesteatoma was non-dependent soft tissue mass alone and/or bony erosion or smooth bony expension was present in all these cases. The distribution of these findings in the various designated regions of the middle ear cavity is given in the table below.
Computerized Tomographic findings
Table X: Extent of the Disease
|Extent||Soft tissue density mass||Soft tissue density mass + bony erosion||Total||Percentage (%)|
|Mastoid Air cells||1||18||19||76%|
On HRCT, epitympanum and antrum were the commonest sites of cholesteatoma, 88% each. Aditus was involved in 21 (84%) cases and posterior tympanum in 13 (52%) cases. 11 (44%) of the cases had involvment of mesotympanum and hypotympanum and 9 (36%) cases had involvment of protympanum. Peril abyrinthine cells were involved in 6 (24%) cases.
Computerized Tomographic findings
Table XI: Involvement of Ossicles
No. of Patients
Destruction of handle of malleus could be identified in 13 (52%) cases and involvement of head of malleus was seen in 11 (44%) cases. Incus was eroded in 15 (60%) cases. However, visualization of stapes was inconsistent with both axial and coronal planes.
Computerized Tomographic findings
Table XII: Complication of cholesteatoma
No. of Cases
|Facial Canal Dehiscnece||4||16%|
|Lateral semincircular canal erosion||2||8%|
|Mastoid Cortex dehiscence||3||12%|
|Sinus plate dehiscence||1||4%|
|Dural plate dehiscence||0||0&|
Preoperative HRCT diagnosed dehiscence of horizontal segment of facial canal in 4 (16%) cases. 2 (8%) cases had erosion of lateral semicircular canal and mastoid cortex dehiscence was seen in 3 (12%) cases. No patient had dural plate dehiscence and only one patient (4%) had sinus plate dehiscence.
Table XIII: Distribution by microorganisms in ear discharge (n = 25)
No. of Patients
Commonest organisms isolated from ear discharge were Staphylococcus aureus (24%) followed by B haemolytic Streptococci (16%), P. aeruginosa (16%) and Acinobacter (16%). 8% of the cases grew H. influenzae. It was noticed that 8% of the cases had mixed flora and 12% of the cases did not grow any organism (Fig. 6).
Table XIV: Distribution of Surgical findings
No. of Patients
|Cholesteatoma + Granulations||3||12%|
|Polyp + Cholesteatoma||1||4%|
On surgical exploration, commonest pathology was cholesteatoma. 3 (12%) cases had only granulations. 3 (12%) cases had both cholesteatoma and granulations. One (4%) patient had polyp and one patient (4%) mucosal hypertrophy only (Fig. 7).
Table XV: Extent of Ear disease
No. of Cases
|Attic + Aditus + Antrum||11||44%|
|Attic + Aditus + Antrum + Mesotympanum||10||40%|
|Others: Mucosal hypertrophy in attic||1||4%|
84% of the patients had extensive disease with 44% of these patients had disease involving antrum, aditus and attic and remaining 40% had involvement of mesotympanum in addition to these. 4% of the patients had involvement of attic only. Cholesteatoma was found in attic and aditus in 8% of cases and only in antrum in 4% of the cases. One patient had only mucosal hypertrophy in attic (Fig. 8).
Table XI: Ossicular involvement
No. of Patients
On surgery it was noticed that incus (68%) was frequently involved followed by head of the malleus (64%). Handle of malleus was necrosed in 15 (60%) cases. Only 6 (24%) had necroses of stapes suprastructure.
Table XVII: Histopathology
No. of Patients
Histopathology diagnosed cholesteatoma in 21 (84%) cases and granulations in 3 (12%) cases. One case had an inflammatory polyp.
Table XVIII: Correlation of CT scan and operative findings
Cases in Agreement
|Evidence of Cholesteatoma||23||21||21||2||1||95||75|
The HRCT found to be very sensitive (95%) in diagnosing cholesteatoma accurately, however HRCT could not differentiate cholesteatoma from granulations and therefore less specific (75%)
Table XIX: Extent of the disease
|Extent||CT Scan||Surgery||Cases in Agreement||False +VE||False -VE||Sensitivity||Specificity|
|Mastoid air cells||19||21||19||0||3||86||100|
The findings on CT i.e. presence of soft tissue density mass with or without bony erosions in the various designated regions, was compared with the operative findings for presence of cholesteatoma. Table 18 shows the distribution of these findings. The widening of,the aditus together with presence of soft tissue density mass was indicative of a presence of cholestetoma in that region. However, in the peril abyrinthine area, erosion of the cells alone was taken as an indicator of cholesteatoma. CT was 100% sensitive for cholesteatoma in protympanum, posterior tympanum, hypotmpanum and peril abyrinthine cells and sensitivity varied from 95% to 86% for other regions. CT was 100% specific for cholesteatoma in epitympanum and mastoid air cells and specificity varied from 86% to 66% for other regions (Fig. 9).
Table XX: Ossicular destruction
|Findings||CT Scan||Surgery||Cases in Agreement||False +VE||False -VE||Sensitivity||Specificity|
CT diagnosed erosion of handle of malleus accurately in 13 of the 15 cases, who found to have erosion on surgery (Sensitivity 81 % and specificity 86%). It failed to identify the erosion of the head of the malleus in'4 of the 16 cases (sensitivity 73% and specificity 100%). CT diagnosed erosion of incus accurately in 14 of the 17 cases and failed to identify erosion in three cases and there was false positive interpretation in one case (Sensitivity 82% and specificity 87%) (Fig. 10).
Table XXI: Correlation of CT scan and operative findings
|Findings||CT Scan||Surgery||Cases in Agreement||False +ve||Fasle -ve||Sensitivity||Specificity|
|Facial Canal Dehiscence||4||3||2||2||1||60||90|
|Lateral Semicircular Canal||2||1||1||1||0||100||94|
|Mastoid Cortex Dehiscence||3||4||3||0||1||75||100|
|Sibus plate dehiscence||1||1||1||0||0||100||100|
CT diagnosed dehisense of horizontal segment of facial canal accurately in 2 cases. There was false positive interpretation in two cases and it failed to identify the dehisence (sensitivity 60% and specificity 90%). It was 100% sensitivity and 95% specific for diagnosis of erosion of LSCS with only one false positive interpretation. Ct diagnosed mastoid cortex dehisence accurately in 3 cases and failed to identify in one case (sensitivity 75% and specificity 100%). It was 100% sensitive and specific for sinus plate dehisence (Fig. 11).
1. The study of clinicopathological and radiological spectrum of cholesteatoma in children
This study was conducted to analyse the clinico-pathological and radiological spectrum of cholesteatoma in children, and to correlate the computed tomographic findings with that of surgical findings in them.
This study included 25 children under the age of 14 years with the clinical diagnosis of chronic suppurative otitis media and cholesteatoma. The upper age limit used by Palva et all (7) Smyth (20) , Glasscock et al (43), Wullstein (44), Abramson et al 45 , and Eldestein et al 46 , varied from 9 to 18 years. The commonest age group in our patients was 11. 1-14 years (52%). There were 14 boys and 11 girls. This male predominance has been observed by all the above authors. Left ear (52%) was more commonly involved than right ear (28%) and 5 (20%) patients had bilateral ear disease. In Glasscock et a1 (43) and Eldestein et a1 (46) series the right ear was predominantly involved.
Commonest complaints were otorrhoea (100%) followed by hearing loss (88%), tinnitus (12%) and vertigo (4%). These results are comparable to the studies done by Palva et all (7) Glasscock et a1 (43), Eldestein et a1 (46) and Triglia (47) . In addition 4 (16%) of our patients presented with post-aural abscess and pain.
The average duration of complaints was 6.9 years, slightly higher than that of results reported by Palva et all (7) , i.e., 5.8 years. This he compared with that of adults, which was 21.8 years and opined that, the cholesteatoma forming mechanism once triggered has a more active course in children.
Preoperative microscopic examination of the ear revealed a variety of abnormalities. The presence of retraction pocket, mainly in the posterosuperior region was the commonest finding (48%). 24% of the patients had attic retraction. 32% of the patients had perforations in the tympanic membrane. These findings are in agreement with a study of 125 cases of childhood cholesteatoma by Eldestein et a1 (46) . Similar to this (46) study cholesteatoma was visualized in 84% of the cases and commonest sites of cholesteatoma were in posterosuperior and attic region. Two (8%) patients had sagging posterior canal wall. One patient had an operated cavity and other one case, a polyp filling external auditory canal.
All patients had conductive hearing loss except one patient, who had a mixed hearing loss with 30 dB AB gap. The common range (44%) of hearing loss was of moderate degree 48 with 30-40 dB AB gap, Glasscock et a1 (43) had similar results in their series of 41 children.
In comparison to studies reported by Palva et a1 (7) , on bacteriological examination of ear discharge, Staphylococcs aureus and Ps. aeruginosa were commonest organisms isolated. Unlike his reports of proteus and E. coli, other organisms isolated were Streptococci and Acinobacter. 2% of our patients had H. influenza. However, percentage of patients whose pus was sterile or grew mixed flora was similar to studies by Palva et al (7).
In the conventional lateral oblique views (Law's projection) 15 (60%) patinets showed sclerotic mastoids. 9 (36%) patients had lytic area in the region of the mastoid antrum which was interpreted as positive for the presence of a cholesteatoma. One patient had an operated cavity. In a study of 95 patients of childhood cholesteatoma by Eldestien et a1 (46) , radiographically the mastoids in the children with acquired cholesteatoma were reported as pneumatized in 54%, one half of these having clouding of the air cells. In 46% the mastoids was observed to be sclerotic.
All patients underwent modified radical mastoidectomy except one patient, in whom intact canal wall masttoidectomy was done. Commonest pathology was cholesteatoma (88%). 12% had only granulations. Granulations were associated with cholesteatoma in 12% of the cases and a polyp in 4% of the cases. One patient had only mucosal hypertrophy. 21 (84%) cases had an extensive disease, 11 (44%) of them had disease involving attic, aditus and antrum and remaining 10 (40%) of them had involvment of middle ear in addition to these. Such extensive findings were also observed by Jhankel et a1 (4), Palva et all (7) , Glasscock et a1 (43) , Schloss et a1 (49), Zorita et a1 (50), Rosenfield et al (51) and Arriaga (52) , while Sheehy (1) and Tos (2) found it to be a less expansive disease. In our study, ossicular involvement was seen in 18 (72%) cases. 16 (64%) patients had multiple ossicular involvement. Incus was commonly involved (68%) followed by head of the malleus (64%) and handle of the malleus (60%). Stapes suprastruture was involved in 24%. These findings are in agreement with studies by Tos (2) , Jhanke et a1 (4) , Palva (7) and Eldestein (46). However Armstrong (41) had contradicting results with less involvement of ossicles.
The horizontal segment of facial canal was dehiscent in 3 (12%) patients. However, neither the disease, nor the surgery to eradicate it produced facial nerve weakness or paralysis. Erosion of the lateral semicircular canal was observed in only one (4%) patient. 4 (16%) patients had destruction of the mastoid cortex and only one (4%) patient had sinus plate destruction. These complications are in comparison to study reported by Eldestein et a1 (46). However, we did not have any patient with erosion of the tegmen.
Histopathological examination revealed cholesteatima in 21 (84%) cases, granulations in 3 (12%) cases and polyp in one (4%) case.
All patients were followed up regularly in ENT outpatient clinic after six and twelve weeks. After six weeks all patients complained of minimal ear discharge except one patient who had a completely dry ear. However, after twelve weeks, 21 of these patients had dry ears with epithelializing cavity. Remaining four patients continued to discharge even after twelve weeks. However, these patients are needed to be followed up reguarly, as the rate of recidivism and recurrence rate reported to be higher in children. Sheehy (1) and Glasscock (43) reported 25% and 5% of recidivism with canal wall up technique and Austin 53 reported 4% recidivism with canal wall down technique. Smyth (20) and Gristwood et a1 (54) propose that the recidivism is proportional to the duration of the follow up. Two potential technologic aids can assist the surgeon in this. They are HRCT and endoscopy. Eldestine and Parisier (10) have advocated serial CT evaluation to detect possible residual disease and aviod second look procedures.
2. Correlation of computed tomographic findings with surgical findings in children
Preoperative CT scan could diagnose non-dependent soft tissue density mass in 23 of the 25 cases (92 percent), with two false positive and one false negative interpretation. On the whole CT was 92% sensitive and 66% specific in identifying soft tissue density mass. Mafee (55) and O'Reilly (56) have similar results, whereas Jackler (57) and Garber (58) found it to be less sensitive and specific. However, CT scan is less sensitive in differentiating cholesteatoma from granulations. Most authors are in agreement with this finding (55, 56, 57 & 58) However Mafee et a1 (39) believed it was possible to identify cholesteatoma by its low attenuation value and Johnson et a1 (59) found that the presence of a well defined edge to mass was a sure indication of cholesteatoma.
Bony erosion, an additional sign for the presence of cholesteatoma was identified in 20 of the 24 cases. This is comparable to the reports by Jackler et a1 (57) and O'Denoghue et a1 (60) , who found cholesteatoma to be present in 80% of the cases with bony erosion who were explored. Using the same criteria O'Reilly (56) detected 23 out of 29 cases of cholesteatoma (79%). Mafee et a1 39 found bone destruction in 9 out of 9 cases of acquired cholesteatoma.
CT was 100% sensitive for cholesteatoma in protympanum, posterior tympanum, hypotmpanum and peril abyrinthine cells and sensitivity varied from 95% to 86% for other regions. CT was 100% specific for cholesteatoma in epitympanum and mastoid air cells and specificity varied from 86% to 66% for other regions. This is in comparision to Garber et al (58) who did similar study.
We found CT to be most accurate in identifying ossicular destruction, which is in consonance with studies by Mafee et a1 (55) , Garber et a1 (58) , Jackler et a1 (57) & Schwartz al (61) . In our study, CT detected ossicular destruction in 15 of the 17 patients (88% sensitivity) who had such lesion on surgery. Mafee et a1 (55) were able to define the state of the ossicular chain in 89% of cases scanned and Jackler et a1 (57) were able to predict the state of the ossicular chain in 83.3% of their cases and O'Rielly (56) could predict an intact ossicular chain correctly in only 50% of the cases. In our study incus was most commonly involved and CT could identify involvement of the incus in 15 of the 17 cases, with destruction found during surgery. Mafee (55) , O'Rielly (56) and Jackler (57) had similar results. O'Donoghue et a1 (60) reported that they had detected erosion of the lung process of the incus in 67% of cases scanned. In our study, erosion of the head of the malleus was found in 16 patients on surgery, however was detected in 11 (73%) patients on CT. CT scan could detect erosion of the handle of the malleus in 13 of the 15 patients who showed this lesion on surgery. This is comparable to studies by Mafee (55) , Garber (58) and Jackler (57). Involvement of the stapes could not be analysed due to inconsistent visualization on CT scan. Most authors had the same problem except O'Donoghue (60) who reported that they could diagnose destruction of stapes suprastructure in 86% of the cases.
Dehiscence of the horizontal part of the facial canal was accurately diagnosed in 3 cases (60% sensitivity and 90% specificity) and there were two false positive interpretations and CT failed to identify the dehiscence in one case. This is in agreement with studies by O'Reilly (56), Jackler (57) and Garber (58). However, Mafee et a1 55 found CT to be very accurate in the diagnosis of erosion of facial canal.
Erosion of the lateral semicircular canal was reported in 3 cases however only one of the patient had a demonstrable lesion on operation. This finding had a sensitivity of 100% and specificity of 94% which is similar to that reported by Mafee et a1 (55).
However, we found CT to be most accurate in diagnosing destruction of mastoid cortex and sinus plate. This is in agreement with studies by most authors.
SUMMARY AND CONCLUSIONS
This study "clinicopathological and radiological spectrum of cholesteatoma in children and correlation of computerized tomographic findings with surgical findings", was conducted in 25 children with chronic suppurative otitis media and cholesteatoma treated in otolaryngological services of PGIMER , Chandigarh and following conclusions were drawn:
a. The study of clinicopathological and radiological spectrum of cholesteatoma in children
Cholesteatoma in children is more aggressive with short course of duration, high incidence of ossicular involvement and other complications and high rate of residual disease.
1. Majority of the patients (52%) were more than 11 years with male predominance (14:11) and left ear (52%) was commonly involved.
2. Commonest complaints were otorrhoea (100%) and hearing loss (88%) and average dutaion of complaints was 6.9 years.
3. Posterosuperior reaction pocket of the pars tensa (48%) was the commonest ear finding followed by attic retraction (24%). 32% of the patients had perforation in the tympanic membrane.
4. Majority (44%) of the patients had moderate degree of the conductive loss.
5. On conventional radiography (Law's projection) 60% of the cases showed sclerotic mastoid and 36% had lytic lesion.
6. 96% of the patients underwent modified radical mastoidectomy and extensive disease was observed in 84% of the patients. Ossicular involvement was seen in 68% of the cases.
7. The horizontal segment of the facial canal was dehiscent in 12% of the patients, erosion of the lateral semicircular canal in 4% of the patients, mastoid cortex dehiscence in 16% and sinus plate destruction in 4% of the cases.
8. After 6 weeks, 96% of the patients had ear discharge and after 12 weeks, 16% of the patients continued to discharge.
b. Correlation of computed tomographic findings with surgical findings in children
The high resolution computerized tomographic scan was both sensitive and specific in diagnosing cholesteatoma, assess extent of the disease, identifying bony erosion and ossicular destruction.
1. The HRCT was 95% sensitive and 75% specific in diagnosing cholesteatoma, but could not differentiate cholesteatoma from granulations.
2. The HRCT was highly sensitive (88%) and specific (97%) in identifying ossicular destruction.
3. The HRCT diagnosed dehiscence of the horizontal segment of the facial canal in 3 cases with two false positive and one false negative interpretations.
4. The HRCT was 100% sensitive and 94% specific in diagnosing erosion of lateral semicircular canal.
5. The HRCT was 75% sensitive and 100% specific diagnosing mastoid cortex and 100% sensitive and specific in diagnosing sinus plate destruction.
Pic. 12: Coronal section CT Scan showing perforation of Tympanic Membrane, normal ossicular mass (Arrow head) and normal "Radiological Spur" (Arrow) i.e., inferior margin of the lateral wall of the attic.
Pic. 13: Coronal section CT Scan showing hypodensity soft tissue mass in the attic (arrow) and head of the Malleus (arrow head).
Pic. 14: Axial section CT Scan showing hypodensity soft tissue mass in the mesotympanum (Arrow) with embeded ossicle remnants (Arrow head).
Pic. 15: Coronal Section CT Scan showing blunting of "Radiological Spur", hypodensity soft tissue mass (Cholesteatoma) in epitympanum, mesotympanum and hypotympanum (Arrow heads).
Pic. 16: Coronal section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in epitympanum, mesotympanum and hypotympanum with widening of the attic (Arrow heads), blunting of radiological spur (asterix) and ossicular remnant.
Pic. 17: Corontal Section CT Scan showing hypodensity soft tissue mass (found to be granulations during surgery), in epitympanum, mesotympanum and hypotympanum with facial canal exposure (Arrow head).
Pic. 18: Coronal Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in hypotympanic recess (Arrow) and erosion of ossicles (Arrow head).
Pic. 19: Coronal Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in epitympanum with erosion of ossicles.
Pic. 20: Coronal Sectiona CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in mastoid antrum with destruction of mastoid air cells, expansion of mastoid antrum (Arrow heads) and mastoid cortex destruction (Arrow).
Pic. 21: Coronal Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in mastoid antrum with destruction of mastoid air cells, expansion of mastoid antrum, large cortex destruction and post-aural abscess (Arrow Head).
Pic. 22: Axial Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in mastoid antrum with destruction of mastoid air cells, expansion of mastoid antrum, destruction of posterior wall of the external auditory canal (Arrow) and destruction of Sinus Plate (Arrow heads).
Pic. 23: Axial Section CT Scan showing hypodensity soft tissue mass ICholesteatoma) in mastoid antrum with destruction of mastoid air cells. expansion of mastoid antrum of destruction of the superior wall of the external auditory canal (Arrow).
Pic. 24: Coronal Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in mastoid antrum with destruction of mastoid air cells, expansion of mastoid antrum and destruction of the superior wall of the external auditory canal (Arrow).
Pic. 25: Coronal Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in mastoid antrum with destruction of mastoid air cells, expansion of mastoid antrum and lateral semicircular cana fistula (Arrow).
Pic. 26: Coronal Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in attic with widening of the attic and lateral semicircular canal fistula (Arrow).
Pic. 27: Axial Section CT Scan showing hypodensity soft tissue mass (Cholesteatoma) in mesotympanum and erosion of horizontal part of the facial canal (Arrow).
Pic. 28: Axial section CT Scan showing large post operative cavity with hypodensity soft tissue mass (Cholesteatoma) in mastoid antrum (Arrow).
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Sr. No. O.C. No.
Tympanic membrane findings
Pars Tens: Type I/II/III/IVC ( Sade's classification)
Pars flaccida: Anterosuperior/Posterosuperior
Mass behind intact drum
Hearing Level: (R) (L)
X-ray mastoid: Pneumatized/Hypocellular/Sclerotic/Lytic lesion
Computerized tomographic findings: (C.T. No. )
Incision Postaural / Endaural / Endomeatal
Antrurn / Attic/ Mesotympanum/ Hypotympanum/ Protympanum/ Ridge/ Sinus tympani/ Perifacial cells/ Facial recess/ Perilabyrinthine cells/ Zygomatic cells/ Tip cells/ Sinudural/ angle/ Dural plate/ Sinus plate/ Lateral semicircular canal/ Facial canal/ Others
Malleus: Head -Intact/necrosed
Incus: Body -
Reconstruction : Tympanoplasty : Nil/I/II/III/IV
Graft used : Temporalis fascia/Perichondrial/skin
Post-op Follow-up: 6 weeks